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Idiopathic hypereosinophilic syndrome (HES) with FIP1L1-PDGFRA rearrangement can be effectively treated with Imatinib

Martinelli, Giovanni, Cilloni, Daniela, Ottaviani, Emanuela, Malagola, Michele, Messa, Francesca, Rondoni, Michela, Bosi, Costanza, Gottardi, Enrico, Rosti, Gianantonio, Ricci, Paolo, Gaitani, Stavroula, Pane, Fabrizio, Testoni, Nicoletta, Mecucci, Cristina, Soverini, Simona, Piccaluga, Pier Paolo, Amabile, Marilina, Tiribelli, Mario, Zaccaria, Alfonso, Grafone, Tiziana, De Vivo, Antonello, Pileri, Stefano, Fattori, Pierpaolo, Bocchia, Monica, Serra, Anna, Maurillo, Luca, Fanin, Renato, Cross, Nicholas C.P., Merante, Serena, Cazzola, Mario, Alimena, Giuliana, Frassoni, Francesco, Galieni, Piero, Russo, Domenico, Gobbi, Marco, Gugliotta, Luigi, Lauria, Francesco, Mazza, Patrizio, Ferrara, Felicetto, Gherlinzoni, Filippo, Leoni, Pietro, Musto, Pellegrino, Baccarani, Michele and Saglio, Giuseppe (2004) Idiopathic hypereosinophilic syndrome (HES) with FIP1L1-PDGFRA rearrangement can be effectively treated with Imatinib Blood, 104, (11), p.421A.

Record type: Article


We studied 141 patients with HES, of which 55 primary HES (39%) defined as a peripheral blood eosinophilia greater than 1,500 cells/µL for longer than 6 months, absence of other apparent aetiologies for eosinophilia and symptoms of organ involvement. All patients were studied by molecular analysis for PDGFRB-TEL, FGFR1-BCR and BCR-ABL transcripts, frequently associated with HES/CMML/MDS syndrome: all these transcripts were absent in our series. We also sought for the recently reported involvement of PDGFR, cryptically translocated with FIP1L1 in some HES pts responsive to Imatinib therapy: 13 pts (23%) were positive for the FIP1L1-PDGFRA rearrangement and all of them showed previously unreported, abnormal-sized fusion transcripts. Curiously, all FIP1L1-PDGFRA positive pts were male. We enrolled in a national clinical trial 31 (55%) primary HES pts, including all 13 (23%) FIP1L1-PDGFRA positive, with imatinib mesylate (100 to 400 mg/day). Median follow up of treatment was 4,5 moths (range 2–28). Rapid and complete haematological responses to imatinib therapy were recorded only in all FIP1L1-PDGFRA positive pts (100%) after one months of therapy and partial response in only one cases with HES without FIP1L1-PDGFRA fusion transcript. Complete molecular response without evidence of FIP1L1-PDGFRA transcript by qualitative RT-PCR was also recorded in all responding pts after median 2 months of therapy. We conclude that FIP1L1-PDGFRA rearrangement may be useful molecular marker of myeloproliferative HES, a predictor of imatinib-responsiveness and as a means to follow therapy in this subgroup of pts.

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Published date: 16 November 2004
Additional Information: ASH Annual Meeting Abstracts, Poster Sessions. Abstract 1504.


Local EPrints ID: 60653
ISSN: 0006-4971
PURE UUID: 37fa4fd5-78f7-4c5b-9244-7326da513b7b
ORCID for Nicholas C.P. Cross: ORCID iD

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Date deposited: 12 Nov 2008
Last modified: 17 Jul 2017 14:23

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Author: Giovanni Martinelli
Author: Daniela Cilloni
Author: Emanuela Ottaviani
Author: Michele Malagola
Author: Francesca Messa
Author: Michela Rondoni
Author: Costanza Bosi
Author: Enrico Gottardi
Author: Gianantonio Rosti
Author: Paolo Ricci
Author: Stavroula Gaitani
Author: Fabrizio Pane
Author: Nicoletta Testoni
Author: Cristina Mecucci
Author: Simona Soverini
Author: Pier Paolo Piccaluga
Author: Marilina Amabile
Author: Mario Tiribelli
Author: Alfonso Zaccaria
Author: Tiziana Grafone
Author: Antonello De Vivo
Author: Stefano Pileri
Author: Pierpaolo Fattori
Author: Monica Bocchia
Author: Anna Serra
Author: Luca Maurillo
Author: Renato Fanin
Author: Serena Merante
Author: Mario Cazzola
Author: Giuliana Alimena
Author: Francesco Frassoni
Author: Piero Galieni
Author: Domenico Russo
Author: Marco Gobbi
Author: Luigi Gugliotta
Author: Francesco Lauria
Author: Patrizio Mazza
Author: Felicetto Ferrara
Author: Filippo Gherlinzoni
Author: Pietro Leoni
Author: Pellegrino Musto
Author: Michele Baccarani
Author: Giuseppe Saglio

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